[Adult onset nemaline myopathy revealed by respiratory insufficiency]. / Révélation d'une myopathie à bâtonnets par une insuffisance respiratoire de l'adulte.

INTRODUCTION: We report a case of nemaline myopathy revealed in adulthood by a respiratory insufficiency. CASE REPORT: A 26-year-old patient, without past history, was admitted with respiratory and right cardiac insufficiency which appeared in a few days. There was a severe restrictive lung impairment with nocturnal hypoventilation. Minor skeletal abnormalities and areflexia suggested a congenital myopathy. Muscle biopsy revealed a nemaline myopathy. CONCLUSION: Respiratory insufficiency is common in nemaline myopathy with infancy or childhood onset, but very rare in adults. It may be explained by multiple mechanisms.

A reappraisal of ICU and long-term outcome of allogeneic hematopoietic stem cell transplantation patients and reassessment of prognosis factors: results of a 5-year cohort study (2009-2013).

Epidemiology and prognosis of complications related to allogeneic hematopoietic stem cell transplant (HSCT) recipients requiring admission to intensive care unit (ICU) have not been reassessed precisely in the past few years. We performed a retrospective single-center study on 318 consecutive HSCT patients (2009-2013), analyzing outcome and factors prognostic of ICU admission. Among these patients, 73 were admitted to the ICU. In all, 32 patients (40.3%) died in ICU, 46 at hospital discharge (63%) and 61 (83.6%) 1 year later. Survivors had a significantly lower sequential organ failure assessment (SOFA) score, serum lactate and bilirubin upon ICU admission. Catecholamine support, mechanical ventilation (MV) and/or renal replacement therapy during ICU stay, a delayed organ support and an active graft versus host disease (GvHD) significantly worsen the outcome. By multivariate analysis, the worsening of SOFA score from days 1 to 3, the need for MV and the occurrence of an active GvHD were predictive of mortality. In conclusion, the incidence of HSCT-related complications requiring an admission to an ICU was at 22%, with an ICU mortality rate of 44%, and 84% 1 year later. A degradation of SOFA score at day 3 of ICU, need of MV and occurrence of an active GvHD are main predictive factors of mortality.

[Right atrial thrombus--a complication of central venous catheters]. / Thrombus auriculaire droit---une complication des voies veineuses centrales.

We describe the development, in three days, of a pediculate mass hanging on the right atrial lateral wall in a 39-year-old woman with a subclavian venous catheterization. She was a current smoker and alcoholic but without drug addict. The hypothesis of a non valvular right atrial infective endocarditis was considered at first, but subsequent events directed the diagnosis towards a thrombus, which was resorbed by heparin. We discuss the incidence, the complications, the treatment and the differential diagnosis of thrombus caused by a central venous catheter. The prevention of right atrial thrombus caused by a central venous catheter depends on the position of the central venous catheter tip, either in the superior vena cava or at the superior vena cava-right atrium junction. A more distal position is a frequent source of thrombotic and embolic complications.

[Pneumococcal infections in intensive care. Retrospective 8 year study]. / Infections à pneumocoques en réanimation. Etude rétrospective sur 8 ans.

OBJECTIVE: The aim of this study was to analyze the clinical presentations and severity of S. pneumoniae infections requiring hospitalization in an intensive care unit and evaluate the incidence and severity of infections caused by penicillin-resistant strains. PATIENTS AND METHODS: This retrospective study reviewed cases in our intensive care unit from January 1989 through December 1996 including all patients with pneumococcal infection. RESULTS: The study included 102 patients, mean age 59.6 years. Pneumonia was the most frequent (83 cases) followed by bacteriemia (31 cases) and meningitis (15 cases). Mortality was high (43%) and influenced by age, simplified severity score, and presence of shock at admission. Antibiotic resistance appeared in 1991 and increased over the years reaching, in 1996: 24% for penicillin, 38% for macrolides, 20% for sulfamides, 19% for tetracyclins, and 14% for phenicols. Penicillin-resistance was not found to modify clinical expression nor severity of infection. Amoxicillin and third-generation cephalosporins were the most widely used antibiotics. CONCLUSION: Pneumococcal infections in intensive care patients are severe with high mortality. The emergence of more and more resistant strains has little clinical consequence on severity or treatment.

[Descending necrotizing mediastinitis. A diagnosis not to be ignored]. / La médiastinite descendante nécrosante. Un diagnostic à ne pas méconnaître.

BACKGROUND: Acute descending posterior mediastinitis is a very serious condition which can develop after common ear-nose-throat infections. Clinical manifestations are typical and must be recognized rapidly for early diagnosis. CASE REPORTS: We report two cases. In the first case, a 28-year-old man had a retropharyngeal abscess which fistulized into the left pleural cavity. Three operations were necessary to achieve cure and favorable outcome. In the second case, mediastinitis was diagnosed in a 39-year-old patient following a throat infection. Despite early surgery, outcome was fatal due to development of pericarditis and tamponnade. DISCUSSION: These two cases illustrate the variable course of descending mediastinitis and emphasize the importance of early medicosurgical cure. Treatment is based on intravenous antibiotics using a combination of 2 or 3 drugs at high doses in association with emergency surgery and extensive mediastinal washings. Despite well-conducted treatment, descending necrotizing mediastinitis may lead to a fatal outcome.

Acquired haemophilia in the elderly is a severe disease: report of five new cases.

Acquired haemophilia is a rare but life-threatening bleeding disease that can be observed in males or females at various ages. In the present study, we report on five cases of acquired factor (F) VIII inhibitors diagnosed in the elderly population over a period of 5 years between 1995 and 1999 in our hospital. The median age of the patients at the time of diagnosis was 76.2 years (66-92 years). In all cases, the diagnosis was suggested by mild to severe bleeding with no previous bleeding history. While the absence of associated conditions is frequently reported especially among the elderly, in our series an underlying disease was found in four out of the five cases: kidney tumour (two cases) and autoimmune disease (two cases). The bleeding was controlled in four patients using porcine FVIII (two cases) or recombinant FVIIa (two cases). The inhibitors were completely resolved in two patients (kidney tumour, GoodPasture syndrome) by treatment of the underlying disease. However, three patients died as direct or indirect consequence of having an inhibitor. Our series confirms and extends previous data reporting the complexity and severity of this disorder. Because bleeding is often severe, a prompt and correct diagnosis is required to provide adequate therapeutic options that take the advanced age of the patients into account.

Hemodynamic effects of 0.375% versus 0.25% bupivacaine during cervical epidural anesthesia for hand surgery.

BACKGROUND AND OBJECTIVES: Cervical epidural anesthesia with 0.375% or 0.25% bupivacaine plus fentanyl is a reliable technique for surgical restoration of digital motion-after tourniquet release and rehabilitation. The study was designed to assess the hemodynamic effects of this technique in 11 ASA 1 patients. METHODS: The epidural catheter was introduced at the C7-T1 interspace on the day of operation. The volume of 0.375% bupivacaine necessary to block the brachial dermatomes was determined. The day after (day 1), every patient received epidurally the determined volume of 0.375% bupivacaine plus 1 microgram/kg fentanyl (group A). On day 2 the same volume of 0.25% bupivacaine plus 1 microgram/kg fentanyl (group B) was injected. For each patient one or several pairs of injection (A + B) were performed in relation with duration of rehabilitation. Cardiac index, stroke volume index, end diastolic index, ejection fraction, and systemic vascular resistance were studied by thoracic electrical bioimpedance. These parameters, mean arterial pressure and heart rate were recorded before and after injection. Sensory level was assessed by loss of cold sensation. RESULTS: Nineteen paired injections were performed. Mean volume of bupivacaine was 7.1 +/- 2 mL. The caudad sensory level was lower in group A: T7 (T3-L1) versus T6 (T2-T11) in group B. Hemodynamic variables were not different between the 2 groups. Mean arterial pressure cardiac index, heart rate, stroke volume index, and ejection fracture decreased slightly as end diastolic index remained unchanged and systemic vascular resistance increased slightly. No correlation was found between hemodynamic changes and spread of analgesia. CONCLUSIONS: Hemodynamic effects, in ASA 1 patients, are moderate and not dependent on the studied concentration of bupivacaine, indicating that a similar degree of sympathetic block is achieved with 0.375% and 0.25% bupivacaine.